Churg-Strauss syndrome (CSS) is a disorder characterized by hypereosinophilia and systemic vasculitis complicating a preexisting asthma. We report here a case of CSS with an endobronchial lesion, initially considered to be an endobronchial granuloma of CSS, which was finally diagnosed as a lipoma, a very rare benign tumor of the tracheobronchial tree. To our knowledge, this is the first case in the literature presenting with these two rare entities.